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Etiopathogenic Role of B Cells in Primary Sjögren’s Syndrome

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Sjögren’s Syndrome

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Abstract

T cells have long dominated the debate on the type of lymphocytes favoring the development of Sjögren’s syndrome (SjS), but in recent years it has become apparent that B cells are also a major contributor to autoimmunity. Beyond the paradigm that T lymphocytes exert control over B lymphocytes, it is now recognized that B cells solicit their own help from T cells, release a flurry of cytokines, and serve as antigen-presenting cells. In SjS, excess of the B-cell activating factor (BAFF) promotes quantitative B-cell anomalies that include an increase in the number of mature B (Bm)2/Bm2’ cells in the circulation and the accumulation of transitional, type 2 marginal zone (MZ) and memory B cells in the target tissues. B cells from SjS patients also display qualitative inconsistencies, such as an abundant local synthesis of BAFF and a default in the mechanism that discards the autoantibody-making B cells within ectopic germinal centers or MZ B-cell aggregates. In short, it is abundantly clear that B cells are involved in the pathogenesis of SjS. Moreover, B-cell-directed treatments have already shown substantial benefits in some SjS subsets.

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Acknowledgments

We are grateful to Geneviève Michel and Simone Forest for cheerful and expert secretarial assistance.

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Authors and Affiliations

  1. EA 2216 “Immunology and Pathology” and IFR 148 ScInBioS, the European University of Brittany and the University of Brest, and the Brest University Medical School Hospital, Brest, France

    Jacques-Olivier Pers, Sophie Hillion, Gabriel Tobón, Valérie Devauchelle, Alain Saraux & Pierre Youinou

  2. Laboratory of Immunology, Brest University Medical School Hospital, Brest, France

    Pierre Youinou

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  1. Jacques-Olivier Pers
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  2. Sophie Hillion
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  3. Gabriel Tobón
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  4. Valérie Devauchelle
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  5. Alain Saraux
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  6. Pierre Youinou
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Editors and Affiliations

  1. Barcelona, Surgey, Hospital Clinic, Calle Villarroel, 170, Barcelona, 08036, Spain

    Manuel Ramos-Casals

  2. Massachusetts General Hospital, Boston, 02114, Massachusetts, USA

    John H. Stone

  3. National University of Athens, Athens, Greece

    Haralampos M. Moutsopoulos

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Pers, JO., Hillion, S., Tobón, G., Devauchelle, V., Saraux, A., Youinou, P. (2011). Etiopathogenic Role of B Cells in Primary Sjögren’s Syndrome. In: Ramos-Casals, M., Stone, J., Moutsopoulos, H. (eds) Sjögren’s Syndrome. Springer, London. https://doi.org/10.1007/978-0-85729-947-5_5

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